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36105

Sigma-Aldrich

(±)-Sodium β-hydroxyisobutyrate

≥96.0%

Synonyme(s) :

β-Hydroxyisobutyric acid sodium salt, (±)-β-HIBA-Na, 3-Hydroxy-2-methylpropionic acid sodium salt, Sodium 3-hydroxy-2-methylpropionate

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About This Item

Formule empirique (notation de Hill):
C4H7NaO3
Numéro CAS:
1219589-99-7
Poids moléculaire :
126.09
Numéro MDL:
MFCD16875405
Code UNSPSC :
12352106
ID de substance PubChem :
329755270
Nomenclature NACRES :
NA.25

Niveau de qualité

100

Pureté

≥96.0% (GC)
≥96.0%

Forme

powder

Composition

sodium, 17.5-19.0%

Température de stockage

2-8°C

Chaîne SMILES 

[Na+].CC(CO)C([O-])=O

InChI

1S/C4H8O3.Na/c1-3(2-5)4(6)7;/h3,5H,2H2,1H3,(H,6,7);/q;+1/p-1

Clé InChI

RBJZIQZDAZLXEK-UHFFFAOYSA-M

Application

3-Hydroxyisobutyrate (HIBA) may be used to study the distribution, characterization and kinetics of enzymes involved in branched chain amino acid metabolism such as 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4).

Actions biochimiques/physiologiques

3-Hydroxyisobutyrate (HIBA), is formed in the valine catabolism, it is a reactant of enzyme 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4); it was found to have increased concentrations in MS patient metabolic profiles.

Conditionnement

Bottomless glass bottle. Contents are inside inserted fused cone.

Code de la classe de stockage

11 - Combustible Solids

Classe de danger pour l'eau (WGK)

WGK 3

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

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Disorders of the metabolism of amino acids and related compounds.
Shih, V. E. and Mandell, R. et al.
Genetic Disorders and the Fetus, 514-553 (2010)
Norbert W Lutz et al.
Biochemical and biophysical research communications, 354(1), 160-164 (2007-01-16)
(1)H NMR spectroscopy of cerebrospinal fluid (CSF) is currently being used to study metabolic profiles characteristic of distinct multiple sclerosis (MS) manifestations. For select MS patient groups, we have previously detected significantly increased concentrations of several identified metabolites and one
Mammalian 3-hydroxyisobutyrate dehydrogenase.
J W Hawes et al.
Methods in enzymology, 324, 218-228 (2000-09-16)
Radovan Murín et al.
Journal of neurochemistry, 105(4), 1176-1186 (2008-02-21)
The branched-chain amino acids (BCAAs)--isoleucine, leucine, and valine--belong to the limited group of substances transported through the blood-brain barrier. One of the functions they are thought to have in brain is to serve as substrates for meeting parenchymal energy demands.
Ference J Loupatty et al.
American journal of human genetics, 80(1), 195-199 (2006-12-13)
Only a single patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency has been described in the literature, and the molecular basis of this inborn error of valine catabolism has remained unknown until now. Here, we present a second patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency
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